Short-term add-on tocilizumab and intravenous cyclophosphamide exhibited a remission-inducing effect in a patient with systemic lupus erythematosus with refractory multiorgan involvements including massive pericarditis and glomerulonephritis

Abstract

We report on a 41-year-old woman with refractory systemic lupus erythematosus with massive pericarditis, macrophage activation syndrome, and glomerulonephritis despite high-dose glucocorticoids and tacrolimus. Tocilizumab dramatically improved pericarditis, and glomerulonephritis was controlled after adding cyclophosphamide. We had to halt tocilizumab and cyclophosphamide due to possible pneumocystis infection after five and three infusions of tocilizumab and intravenous cyclophosphamide, respectively. Nevertheless, no lupus flare had been observed on glucocorticoid monotherapy and enabled further rapid tapering prednisolone.

Effect of Biologic Therapy on Clinical and Laboratory Features of Macrophage Activation Syndrome Associated With Systemic Juvenile Idiopathic Arthritis

“…This raises the possibility that cytokine‐directed treatment with biologic agents could serve as an effective treatment for MAS. Indeed, there are several reports of MAS treated with high doses of anakinra , and scattered reports of treatment with tocilizumab . However, this systematic review demonstrates that MAS also occurs with regularity on these biologic agent treatments and therefore suggests that other pathways may be more central to MAS pathogenesis in various patient populations.…”

The regulation of the Treg/Th17 balance by mesenchymal stem cells in human systemic lupus erythematosus

“…14 However, an IL-17 inhibitor has not been considered for the clinical treatment of lupus patients. 15 An IL-6 receptor antagonist, tocilizumab, induced short-term disease remission in some cases of lupus nephritis 16,17 but with high rate of relapse after long-term follow-up, 18 suggesting that directly inhibiting Th17 cells and its related cytokines was not appropriate for lupus treatment. A recent study by Schmidt et al showed that IL-17A deficiency did not affect the morphological or functional parameters in MRL/lpr mice with lupus nephritis, nor did IL-17A neutralization affect the clinical course of nephritis in NZB/NZW mice, suggesting that the Th17/IL-17A immune response plays no major role in the immunopathogenesis of lupus nephritis in MRL/lpr and NZB/NZW mice.…”

Reduced Let-7f in Bone Marrow-Derived Mesenchymal Stem Cells Triggers Treg/Th17 Imbalance in Patients With Systemic Lupus Erythematosus

“…IL-6 induces the expression of IL-23R and RORγt, thus promoting the generation of Th17 cells from naïve T cells together with TGF-β while inhibits TGF-β induced Treg (iTreg) differentiation (33). It has been reported that IL-6 levels are increased in various autoimmune diseases including rheumatoid arthritis (RA), multiple sclerosis (MS) and lupus (34), and targeting IL-6 can be an effective approach in the treatment of several autoimmune diseases (35,36). Here we demonstrated for the first time that increased IL-6 might be induced by impaired let-7f in SLE BM-MSCs, which might led to IL-6-STAT3-NF-κB pro-inflammatory circuit, and thus contribute to lupus development.…”